Pleomorphic sarcomas or spindle cell sarcomas of the bone are rare malignant tumors that affect the adult. Fracture in the affected bone is the most frequent first symptom. Multimodal treatment is similar to the one for osteosarcoma, but the benefit is less evidence-based, due to the rarity of this sarcoma subtype. The present paper is a case report of a 73-year-old patient, who presented with a comminuted fracture of the distal third of the femur after a fall on ice. Differential diagnosis of bone metastasis was made. After the histopathological confirmation, the surgical team decided to amputate, with prior patient consent. The tumor was staged pT1NxMx, R0. Immunohistochemical studies confirmed the histopathological diagnosis. A lytic lesion in the stump bone appeared on post-operative MRI and was interpreted as skip metastasis. No other metastatic sites were detected. The multidisciplinary team decided for adjuvant chemotherapy (3 courses) and then radiotherapy. He was unable to receive the total planned dose of radiotherapy due to local toxicity. Even so, he is free of recurrence on long-term follow-up.
Myxofibrosarcoma or myxoid malignant fibrous histiocytoma is one of the most common sarcomas of the limb. It is usually treated multimodally. Most frequent sites of metastasis are the bone, lung and lymph nodes. The present paper is a case report of a 65-year-old male with myxofibrosarcoma of the fibularis longus muscle, for which he first underwent surgery - tumor resection with appropriate margins. The tumor was staged pT2b cN0 cM0. Postoperative PET-CT revealed metabolically inactive pulmonary nodules. Two months after surgery, he underwent adjuvant radiotherapy, a total dose of 60 Gy and 6 courses of chemotherapy (doxorubicin and ifosfamide). Pulmonary nodules have been stationary on all subsequent imagistic studies. He is free of recurrence on long-term follow-up.
Introduction. Skin toxicity in patients receiving novel therapeutic cancer agents has become a very important marker in determining drug activity, but it can also severely impact their quality of life. About half of the patients receiving this type of oncologic treatment will develop cutaneous reactions, that is why adequate understanding and management of these side effects is very important for drug adherence and patients’ quality of life.
Materials and methods. We conducted a prospective study of consecutive patients who received oncologic treatment in our institution and presented with dermatologic side effects. The severity of skin toxicity was assessed using the DLQI score and patients were prospectively followed to evaluate response to therapy. Univariate analysis of factors influencing the impact of skin toxicity on patient QOL was conducted.
Results. 52 patients were enrolled in the study. Patients who developed grade 3 and 4 skin toxicity had a higher DLQI score, with a greater impact on quality of life, but with better clinical outcome at 3 months follow-up, based on RECIST. Patients with moderate or severe cutaneous AE were more likely to achieve complete or partial response to therapy than those with mild AE (16/33 vs. 3/19, p = 0.035). Interestingly, female patients had a significantly poorer quality of life than male patients as assessed by the DLQI score (7.28 ± 7 vs. 3.7 ± 3.6, p = 0.038).
Conclusion. Cutaneous side effects are often encountered in cancer patients and their severity can be a surrogate marker for a positive clinical tumor response to therapy.
Malignant peripheral nerve sheath tumors are a type of soft tissue sarcoma deriving from Schwann cells that usually appear in type 1 neurofibromatosis patients but also sporadically. Tumors frequently interest the nerves in the limbs. They represent a therapeutical challenge due to difficulty of resection and relative radio-resistance and chemo-resistance. The paper aims to describe targeted therapy used in this setting and news concerning the molecular changes that lead to carcinogenesis initiation and promotion. A short selection of literature data was made using PRISMA criteria on this topic. However, due to the rarity and heterogeneity of these tumors, personalized treatment is necessary.
Giant cell tumors of the bone are tumors whose malignant character has long been debated. Lung metastases have been reported in some cases. They usually represent osteolytic, locally aggressive bone tumors for which surgery is the standard of care. Denosumab is the most effective systemic treatment in these cases, but both the timing and the duration are a matter of debate. The aim of this short review was to describe the most important trials that treated patients with this drug and to discuss both advantages and long-term toxicity. It can be concluded from the presented data that the choice of adding denosumab in the treatment sequence of giant cell tumor of the bone has to be taken in a personalized manner for each patient.
The second most frequent malignant tumor of the bone after osteosarcoma, chondrosarcoma is subdivided in conventional type, mesenchymal, clear cell, and the dedifferentiated subtype. Each of these pathological entities has a particular clinical behavior. For most, surgery remains the sole valid option. However, efficient systemic therapy options for advanced and metastatic cases are scarce. This short review is aimed at describing the latest options presented by current literature in these cases. Most of the data is derived from preclinical trials, but some drugs were also included in clinical research as far as phase two trials. After reviewing this data, it could be concluded that the future in unresectable or metastatic chondrosarcoma is personalized medicine and that more specific biomarkers to aid the choice are necessary.